Activity
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| Intellectual disability v8.97 | PI4K2A |
Sarah Leigh Tag Q3_24_promote_green was removed from gene: PI4K2A. Tag Q3_24_NHS_review was removed from gene: PI4K2A. |
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| Intellectual disability v8.97 | PI4K2A | Sarah Leigh reviewed gene: PI4K2A: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.97 | PI4K2A |
Sarah Leigh Source Expert Review Green was added to PI4K2A. Rating Changed from Amber List (moderate evidence) to Green List (high evidence) |
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| Intellectual disability v8.13 | PI4K2A | Achchuthan Shanmugasundram Deleted their comment | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.13 | PI4K2A | Achchuthan Shanmugasundram Classified gene: PI4K2A as Amber List (moderate evidence) | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.13 | PI4K2A | Achchuthan Shanmugasundram Added comment: Comment on list classification: As reviewed by Tracy Lester, there are four unrelated cases reported with global developmental delay and/ or profound intellectual disability. Hence, this gene should be promoted to green rating in the next GMS update. | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.13 | PI4K2A | Achchuthan Shanmugasundram Gene: pi4k2a has been classified as Amber List (Moderate Evidence). | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.13 | PI4K2A | Achchuthan Shanmugasundram Classified gene: PI4K2A as Amber List (moderate evidence) | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.13 | PI4K2A | Achchuthan Shanmugasundram Added comment: Comment on list classification: As reviewed by Tracy Lester, there are four unrelated cases reported with global developmental delay and/ or profound intellectual disability. Hence, this gene should be promoted to green rating in the next GMS update. | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.13 | PI4K2A | Achchuthan Shanmugasundram Gene: pi4k2a has been classified as Amber List (Moderate Evidence). | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.12 | PI4K2A | Achchuthan Shanmugasundram Added comment: Comment on phenotypes: This gene has been associated with relevant phenotype in OMIM (MIM #620732), but not yet in Gene2Phenotype. | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.12 | PI4K2A | Achchuthan Shanmugasundram Phenotypes for gene: PI4K2A were changed from Neurodevelopmental disorder with hyperkinetic movements, seizures and structural brain abnormalities, OMIM:620732 to Neurodevelopmental disorder with hyperkinetic movements, seizures and structural brain abnormalities, OMIM:620732 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.11 | PI4K2A | Achchuthan Shanmugasundram Phenotypes for gene: PI4K2A were changed from Neurodevelopmental disorder with hyperkinetic movements, seizures and structural brain abnormalities, OMIM:620732 to Neurodevelopmental disorder with hyperkinetic movements, seizures and structural brain abnormalities, OMIM:620732 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.11 | PI4K2A | Achchuthan Shanmugasundram Phenotypes for gene: PI4K2A were changed from Neurodevelopmental disorder with hyperkinetic movements, seizures and structural brain abnormalities, OMIM:620732 to Neurodevelopmental disorder with hyperkinetic movements, seizures and structural brain abnormalities, OMIM:620732 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.10 | PI4K2A | Achchuthan Shanmugasundram Phenotypes for gene: PI4K2A were changed from Intellectual disability; developmental delay; seizures to Neurodevelopmental disorder with hyperkinetic movements, seizures and structural brain abnormalities, OMIM:620732 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.10 | PI4K2A | Achchuthan Shanmugasundram Publications for gene: PI4K2A were set to 30564627; 32418222' | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.10 | PI4K2A | Achchuthan Shanmugasundram Publications for gene: PI4K2A were set to 30564627; 35880319; 32418222 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.9 | PI4K2A |
Achchuthan Shanmugasundram Tag Q3_24_promote_green tag was added to gene: PI4K2A. Tag Q3_24_NHS_review tag was added to gene: PI4K2A. |
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| Intellectual disability v8.9 | PI4K2A | Achchuthan Shanmugasundram reviewed gene: PI4K2A: Rating: GREEN; Mode of pathogenicity: None; Publications: ; Phenotypes: Neurodevelopmental disorder with hyperkinetic movements, seizures and structural brain abnormalities, OMIM:620732; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Intellectual disability v8.6 | PI4K2A |
Tracy Lester gene: PI4K2A was added gene: PI4K2A was added to Intellectual disability. Sources: NHS GMS Mode of inheritance for gene: PI4K2A was set to BIALLELIC, autosomal or pseudoautosomal Publications for gene: PI4K2A were set to 30564627; 35880319; 32418222 Phenotypes for gene: PI4K2A were set to Intellectual disability; developmental delay; seizures Penetrance for gene: PI4K2A were set to unknown Review for gene: PI4K2A was set to GREEN Added comment: At least three cases have been reported with biallelic variants in this gene and a neurodevelopmental disorder 35880319 - Two patients with PI4K2A deficiency (homozygous variants) were identified by exome sequencing, presenting with developmental and epileptic-dyskinetic encephalopathy. Neuroimaging showed corpus callosum dysgenesis, diffuse white matter volume loss, and hypoplastic vermis. In addition to NDD, we observed recurrent infections and death at toddler age. 30564627 - We report a family of Saudi Arabian ancestry with two children presenting with global developmental delay, dystonia, disturbed sleep, and heat intolerance. By genome sequencing, we identified a nonsense variant in the first exon of PI4K2A that was homozygous in both affected individuals and was absent from, or heterozygous in, seven unaffected siblings. 32418222 - a homozygous missense variant of uncertain significance was suggested to be responsible for some features in a case with NDD and metabolic cutis laxa. Sources: NHS GMS |
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